Brett Ley
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View article: Telomere length and immunosuppression in non-idiopathic pulmonary fibrosis interstitial lung disease
Telomere length and immunosuppression in non-idiopathic pulmonary fibrosis interstitial lung disease Open
Background Studies suggest a harmful pharmacogenomic interaction exists between short leukocyte telomere length (LTL) and immunosuppressants in idiopathic pulmonary fibrosis (IPF). It remains unknown if a similar interaction exists in non-…
View article: Telomere length associates with chronological age and mortality across racially diverse pulmonary fibrosis cohorts
Telomere length associates with chronological age and mortality across racially diverse pulmonary fibrosis cohorts Open
Pulmonary fibrosis (PF) is characterized by profound scarring and poor survival. We investigated the association of leukocyte telomere length (LTL) with chronological age and mortality across racially diverse PF cohorts. LTL measurements a…
View article: A low-cost, highly functional, emergency use ventilator for the COVID-19 crisis
A low-cost, highly functional, emergency use ventilator for the COVID-19 crisis Open
Respiratory failure complicates most critically ill patients with COVID-19 and is characterized by heterogeneous pulmonary parenchymal involvement, profound hypoxemia and pulmonary vascular injury. The high incidence of COVID-19 related re…
View article: Peripheral blood leucocyte telomere length is associated with progression of interstitial lung disease in systemic sclerosis
Peripheral blood leucocyte telomere length is associated with progression of interstitial lung disease in systemic sclerosis Open
Background Peripheral blood leucocyte telomere length (PBL-TL) is associated with outcomes in patients with idiopathic pulmonary fibrosis. Whether PBL-TL is associated with progression of systemic sclerosis-associated interstitial lung dis…
View article: Leukocyte telomere length and mycophenolate therapy in chronic hypersensitivity pneumonitis
Leukocyte telomere length and mycophenolate therapy in chronic hypersensitivity pneumonitis Open
Recent prospective clinical trials have shown antifibrotic therapies slow lung function decline in patients with idiopathic pulmonary fibrosis (IPF) [1, 2] and progressive fibrosing interstitial lung disease (ILD). Similar findings were de…
View article: A low-cost, rapidly scalable, emergency use ventilator for the COVID-19 crisis
A low-cost, rapidly scalable, emergency use ventilator for the COVID-19 crisis Open
For the past 50 years, positive pressure ventilation has been a cornerstone of treatment for respiratory failure. Consensus surrounding the epidemiology of respiratory failure has permitted a relatively good fit between the supply of venti…
View article: Prognostic value of transbronchial lung cryobiopsy for the multidisciplinary diagnosis of idiopathic pulmonary fibrosis: a retrospective validation study
Prognostic value of transbronchial lung cryobiopsy for the multidisciplinary diagnosis of idiopathic pulmonary fibrosis: a retrospective validation study Open
View article: Impact of Idiopathic Pulmonary Fibrosis on Longitudinal Health-care Utilization in a Community-Based Cohort of Patients
Impact of Idiopathic Pulmonary Fibrosis on Longitudinal Health-care Utilization in a Community-Based Cohort of Patients Open
This study defines a marked increase in HRU in patients with IPF compared with control subjects, with accelerated use beginning at least 1 year prediagnosis and elevated use sustained over the following 5 years. To our knowledge, this is t…
View article: Peripheral Blood Leukocyte Telomere Length Predicts Progression of Interstitial Lung Disease Associated with Systemic Sclerosis: Longitudinal Analysis of an Observational Cohort Study with Independent Validation
Peripheral Blood Leukocyte Telomere Length Predicts Progression of Interstitial Lung Disease Associated with Systemic Sclerosis: Longitudinal Analysis of an Observational Cohort Study with Independent Validation Open
View article: Telomere length in patients with unclassifiable interstitial lung disease: a cohort study
Telomere length in patients with unclassifiable interstitial lung disease: a cohort study Open
Up to 15% of patients with chronic interstitial lung disease (cILD) will remain clinically unclassifiable ( i.e. unclassifiable ILD, uILD) despite thorough clinical evaluation and multidisciplinary team discussion (MDT) [1, 2]. This diagno…
View article: Bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: a systematic review and meta-analysis
Bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: a systematic review and meta-analysis Open
Background The role of bronchoalveolar lavage fluid (BALF) lymphocyte percentage in diagnosing chronic hypersensitivity pneumonitis (CHP) is unclear. We conducted a systematic review and meta-analysis of bronchoalveolar lavage (BAL) lympho…
View article: Corticosteroid use is not associated with improved outcomes in acute exacerbation of IPF
Corticosteroid use is not associated with improved outcomes in acute exacerbation of IPF Open
Background and objective AE‐IPF has profound prognostic implications, preceding approximately half of all IPF‐related deaths. Despite this clinical significance, there are limited data to guide management decisions. Corticosteroids remain …
View article: Risk factors for disease progression in idiopathic pulmonary fibrosis
Risk factors for disease progression in idiopathic pulmonary fibrosis Open
In this retrospective study of a randomised trial of simtuzumab in idiopathic pulmonary fibrosis (IPF), prodromal decline in forced vital capacity (FVC) was significantly associated with increased risk of mortality, respiratory and all-cau…
View article: Concomitant medications and clinical outcomes in idiopathic pulmonary fibrosis
Concomitant medications and clinical outcomes in idiopathic pulmonary fibrosis Open
Patients with idiopathic pulmonary fibrosis (IPF) frequently have a substantial burden of comorbidities [1]. Antifibrotic therapy is recommended to slow the progression of IPF [2]. Patients receiving antifibrotic therapy frequently receive…
View article: Rebuttal From Dr Ley
Rebuttal From Dr Ley Open
View article: COUNTERPOINT: Should Molecular and Genetic Biomarkers Be Used in the Initial Evaluation of Patients With Fibrotic ILD? No
COUNTERPOINT: Should Molecular and Genetic Biomarkers Be Used in the Initial Evaluation of Patients With Fibrotic ILD? No Open
View article: Rare Protein-Altering Telomere-related Gene Variants in Patients with Chronic Hypersensitivity Pneumonitis
Rare Protein-Altering Telomere-related Gene Variants in Patients with Chronic Hypersensitivity Pneumonitis Open
Rationale: Rare genetic variants in telomere-related genes have been identified in familial, idiopathic, and rheumatoid arthritis-associated pulmonary fibrosis. Short peripheral blood leukocyte (PBL) telomere length predicts poor ou…
View article: Pulmonary physiology is poorly associated with radiological extent of disease in systemic sclerosis-associated interstitial lung disease
Pulmonary physiology is poorly associated with radiological extent of disease in systemic sclerosis-associated interstitial lung disease Open
Trials in systemic sclerosis-associated interstitial lung disease (SSc-ILD) typically use forced vital capacity (FVC) as the primary end-point [1, 2]; however, pulmonary function tests have limitations in SSc-ILD, notably being influenced …
View article: 3541 The association of corticosteroid use with inpatient mortality in acute exacerbation of idiopathic pulmonary fibrosis
3541 The association of corticosteroid use with inpatient mortality in acute exacerbation of idiopathic pulmonary fibrosis Open
OBJECTIVES/SPECIFIC AIMS: Objective: To assess the impact of corticosteroid therapy on in-hospital mortality in IPF patients admitted with acute respiratory failure. METHODS/STUDY POPULATION: Methods: Patients with IPF were retrospectively…
View article: Significance of bronchiolocentric fibrosis in patients with histopathological usual interstitial pneumonia
Significance of bronchiolocentric fibrosis in patients with histopathological usual interstitial pneumonia Open
Aims To evaluate the clinical significance of bronchiolocentric fibrosis (BCF) in patients with a histopathological pattern of usual interstitial pneumonia (UIP). Methods and results Two hundred and fifty‐two patients with pathological UIP…
View article: Telomere length and genetic variant associations with interstitial lung disease progression and survival
Telomere length and genetic variant associations with interstitial lung disease progression and survival Open
Leukocyte telomere length (LTL), MUC5B rs35705950 and TOLLIP rs5743890 have been associated with idiopathic pulmonary fibrosis (IPF). In this observational cohort study, we assessed the associations between these genomic markers and outcom…
View article: The added value of comorbidities in predicting survival in idiopathic pulmonary fibrosis: a multicentre observational study
The added value of comorbidities in predicting survival in idiopathic pulmonary fibrosis: a multicentre observational study Open
Background The gender–age–physiology (GAP) model was developed to predict the risk of death. Comorbidities are common in idiopathic pulmonary fibrosis (IPF) and may impact on survival. We evaluated the ability of comorbidities to improve p…
View article: Analysis of pulmonary features and treatment approaches in the COPA syndrome
Analysis of pulmonary features and treatment approaches in the COPA syndrome Open
The COPA syndrome is a monogenic, autoimmune lung and joint disorder first identified in 2015. This study sought to define the main pulmonary features of the COPA syndrome in an international cohort of patients, analyse patient responses t…
View article: Identification of Diagnostic Criteria for Chronic Hypersensitivity Pneumonitis. An International Modified Delphi Survey
Identification of Diagnostic Criteria for Chronic Hypersensitivity Pneumonitis. An International Modified Delphi Survey Open
This consensus-based approach for the diagnosis of cHP represents a first step towards the development of international guidelines for the diagnosis of cHP.
View article: Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case–cohort study
Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case–cohort study Open
We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF e…
View article: The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: an observational cohort-control study
The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: an observational cohort-control study Open
View article: Mortality Risk Prediction in Scleroderma-Related Interstitial Lung Disease
Mortality Risk Prediction in Scleroderma-Related Interstitial Lung Disease Open
View article: Pirfenidone Reduces Respiratory-related Hospitalizations in Idiopathic Pulmonary Fibrosis
Pirfenidone Reduces Respiratory-related Hospitalizations in Idiopathic Pulmonary Fibrosis Open
In a pooled analysis of three phase III IPF clinical trials, patients receiving pirfenidone had a lower risk of nonelective respiratory-related hospitalization over the course of 1 year. The effect of pirfenidone on death after hospitaliza…
View article: The performance of the GAP model in patients with rheumatoid arthritis associated interstitial lung disease
The performance of the GAP model in patients with rheumatoid arthritis associated interstitial lung disease Open
View article: Code-based Diagnostic Algorithms for Idiopathic Pulmonary Fibrosis. Case Validation and Improvement
Code-based Diagnostic Algorithms for Idiopathic Pulmonary Fibrosis. Case Validation and Improvement Open
A well-accepted International Classification of Diseases, Ninth Revision, code-based IPF algorithm performs poorly, falsely classifying many non-IPF cases as IPF and missing a substantial proportion of IPF cases. A modification of the IPF …